What Are The Complications Of Thalassemia?

Thalassemia is a genetic blood disorder that occurs when an individual has less hemoglobin and lower counts of red blood cells in their body than they should. Because red blood cells and hemoglobin are responsible for the delivery of oxygen to all of the tissues around the body, an affected individual will develop anemia and feel frequently fatigued. Thalassemia happens when an individual has mutations in the DNA of the cells responsible for producing hemoglobin. Most of these mutations can be passed from a parent to their child. The majority of thalassemia patients develop symptoms within the first several years of their life. Diagnosis is made upon specific characteristics identified in a blood test. Thalassemia can vary in severity from requiring no treatment to requiring blood infusions or a stem cell transplant. Several possible complications can occur in individuals with untreated thalassemia.

Increased Risk Of Infection

Infection. Photo Credit: Dreamstime @Peter P

A common and dangerous complication that occurs in thalassemia patients is an increased risk of infection. Thalassemia can cause an individual's body to become immunocompromised or a state where the body's infection-fighting defenses are not functioning correctly. The mechanism that causes an immune compromise in these individuals involves the spleen, which sits just under the ribs on the left side of the abdomen. The spleen is responsible for several things including the production of blood cells, monitoring blood for infections, and filtering the blood of wastes. Once the spleen detects an infection in the blood, the individual's body can start the process of fighting it off. However, a thalassemia patient has an overwhelmed spleen. Because the bone marrow cannot make enough healthy blood cells on its own, the spleen tries to compromise by attempting to make more blood cells than it usually would. This effort results in an impairment of the other functions the spleen is responsible for performing including the monitoring of blood for infections. When the spleen is not monitoring as it should be, the patient's body will be prone to invasion by viruses, bacteria, fungi, and parasites that can multiply into a full-blown infection.

Splenomegaly

Photo Credit: KidsKunst

Splenomegaly, an enlarged spleen, is a complication often seen in thalassemia patients. The spleen is responsible for performing numerous functions for the body like producing lymphocytes or white blood cells, filtering and dismantling old or damaged blood cells, and storing platelets and red blood cells. Thalassemia is often accompanied by the demolition of high numbers of red blood cells. The spleen filters these cells from the blood and then removes the iron and globulin from them for reuse. When parts of red blood cells accumulate in the spleen from increased cell destruction, the organ enlarges. Iron also abnormally accumulates in the spleen because the organ is so overwhelmed that it does not carry out the rest of the iron recycling process. At that point, the spleen continues to work hard to filter the large quantities of dying or dead red blood cells, taking in some healthy red blood cells and platelets during the process. This mechanism causes further enlargement of the spleen. This spleen malfunction can even adversely affect the lifespan of healthy red blood cells a patient has received through a transfusion.

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